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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Ann+Dermatol
2015 ; 27
(6
): 727-37
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Blastic Plasmacytoid Dendritic Cell Neoplasm: Analysis of Clinicopathological
Feature and Treatment Outcome of Seven Cases
#MMPMID26719643
Kim JH
; Park HY
; Lee JH
; Lee DY
; Lee JH
; Yang JM
Ann Dermatol
2015[Dec]; 27
(6
): 727-37
PMID26719643
show ga
BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN), which is
derived from the precursor of plasmacytoid dendritic cells, is a rare and highly
aggressive hematologic malignancy. It has only recently been recognized as a
distinct entity. BPDCN characteristically has a predilection for cutaneous
involvement. OBJECTIVE: The aim of this study was to describe the clinical and
pathological features of BPDCN, and to review the treatment courses to analyze
the prognosis and the optimal therapeutic approach. METHODS: We retrospectively
reviewed seven BPDCN cases registered in the Samsung Medical Center database
between January 2010 and December 2014. RESULTS: The median age of the patients
was 52 years (range, 18~79 years), and six patients were male. The clinical
staging was as follows: skin (n=5), lymph node (n=6), bone marrow (n=4), and
peripheral blood (n=2). The skin manifestations were bruise-like tumefaction
(n=4), erythematous nodule (n=4), or multiple erythematous papules (n=1). The
pathological evaluation revealed dense diffuse or nodular infiltration of
neoplastic cells, which were positive for CD4, CD56, and CD123 in the
immunohistochemical analysis. Six patients received multiagent chemotherapy as
the first-line treatment, alone (n=4), or followed by stem cell transplantation
(SCT, n=1) or concurrent radiotherapy (n=1). The median progression-free survival
after the first-line treatment was 6 months (range, 2~12 months). CONCLUSION:
Three different skin manifestations were observed, with pathological features
analogous to each other. All patients who received chemotherapy without SCT
achieved partial or complete response but experienced relapse. Furthermore, they
showed various clinical courses irrelevant to the cutaneous involvement.